Keyword - Pyropoikilocytosis (KW-0668)
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Definition
Protein which, if defective, causes hereditary pyropoikilocytosis (HPP), a recessively inherited hemolytic anemia characterized by microspherocytosis, poikilocytosis (deformation of the erythrocytes), and an unusual thermal sensitivity of red cells. Frequently associated with abnormalities in alpha-spectrin, one of the principal structural proteins of the erythrocyte membrane skeleton.
Synonyms
- HPP
Category
› Disease
Graphical
